J Korean Pediatr Soc.
2000 Mar;43(3):444-449.
A Month-Old Infant with Chronic Granulomatous Disease Presented as Pulmonary Aspergillosis
- Affiliations
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- 1Department of Pediatrics, College of Medicine, Ewha Womans University, Seoul, Korea.
- 2Department of Pathology, College of Medicine, Ewha Womans University, Seoul, Korea.
- 3Department of Microbiology, College of Medicine, Ewha Womans University, Seoul, Korea.
- 4Department of Clinical Pathology, College of Medicine, Ewha Womans University, Seoul, Korea.
- 5Department of Thoracic Surgery, College of Medicine, Ewha Womans University, Seoul, Korea.
- 6Department of Radiology, College of Medicine, Ewha Womans University, Seoul, Korea.
Abstract
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Chronic granulomatous disease (CGD) is an uncommon inherited disorder in which phagocytic cells fails to produce antimicrobial superoxide (O2-) due to NADPH oxidase deficiency. This disease can be easily screened by PMA (phobol myristate acetate)-activated nitroblue tetrazolium (NBT) test. Also the disease has features of chronic pyogenic infection, granuloma formation, anemia, hypergammaglobulinemia, and leukocytosis. Treatment uses antibiotics sensitive to specific organism, surgical drainage of abscess and antifungal drugs for fungal infection. Recently, interferon-gamma (INF-gamma) has been used as treatment and prevention. We experienced a case of CGD with pulmonary aspergillosis in a month-old infant. It was confirmed by activated NBT test. Aspergillus was observed in his lung tissue and Aspergillus fumigatus was cultured from pus of the chest wall. His symptoms improved after antifungal and INF-gamma treatment.